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J Thorac Cardiovasc Surg 2001;121:590-592
© 2001 The American Association for Thoracic Surgery
Brief Communications |
From the Departments of Surgery and Internal Medicine, Hennepin County Medical Center, Minneapolis, Minn.
Received for publication June 21, 2000. Accepted for publication July 12, 2000. Address for reprints: Joseph R. Van Camp, MD, Department of Surgery, Cardiothoracic Section, Hennepin County Medical Center, 702 Park Ave, Minneapolis, MN 55415 (E-mail: joe.van.camp{at}co.hennepin.mn.us).
Primary malignant tumors of the aorta are rare, and primary sarcomas of the aorta are even more so. This report presents a unique case of a myxoid leiomyosarcoma successfully removed from the descending aorta after presentation with mesenteric ischemia. The majority of aortic malignant tumors will reach clinical attention because of tumor embolism. Although cure is unlikely, this case supports an aggressive multidisciplinary approach to the treatment of these rare slow-growing tumors for palliation.
A 48-year-old woman presented with a 2-year history of sharp epigastric pain often radiating to both upper quadrants in conjugation with a 30-lb weight loss. Significant fever, leukocytosis, microcytic anemia, and elevated liver enzyme levels accompanied these episodes and eventually became persistent and unrelenting. The patient underwent cholecystectomy and exploratory laparotomy without resolution. She had donated a kidney to a sibling 18 years before and subsequently had progressive renal insufficiency with a creatinine level of 2.0 mg/dL that delayed obtaining good contrast-enhanced abdominal computed tomographic scans. She was referred for endoscopic ultrasonography to evaluate for possible chronic pancreatitis. This showed a normal pancreas; however, a large intra-aortic mass was seen extending from the mid thoracic aorta to the origin of the superior mesenteric artery, with no flow in the celiac origin on Doppler examination. This was confirmed by magnetic resonance imaging and contrast-enhanced computed tomography, which revealed an irregular filling defect in the mid thoracic aorta, extending into the abdominal aorta, occluding the celiac axis, and terminating 2 cm into the proximal superior mesenteric artery (Fig 1). A single right kidney demonstrated multiple wedge-shaped areas of hypodensity consistent with infarction or embolization, and leg x-ray films revealed multiple lytic lesions in the long bones.
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Subsequent follow-up revealed more metastatic bone lesions in her legs for which she received palliative radiation therapy and internal fixation of the fibula. At 12 months' follow-up, she remained asymptomatic from the primary tumor, and repeat magnetic resonance imaging showed no recurrent aortic mass. She continued to experience significant lower extremity bone pain from slow-growing metastatic lesions; however, she was able to return to work, eat, and gain weight. She ultimately died of cancer at 18 months from intra-abdominal and pelvic metastatic disease.
Primary aortic tumors are rare, with only 42 cases reported in the world literature. It was not until 1960 that the first antemortem diagnosis of an aortic tumor was made in a 22-year-old woman with malignant hypertension and a thoracoabdominal fibromyxoma.
1,2
Morphologically, 3 growth patterns of primary aortic sarcomas have emerged: intraluminal, intimal, and mural or adventitial. Intraluminal tumors, as in this case, are generally polypoid, growing in the direction of vascular flow. They present either with local obstruction to vascular flow or as acute embolic events. 2,3 These tumors are slow growing, as is evidenced in this case, and usually very difficult to diagnose, presenting only in very advanced stages or when tumor embolization causes obstruction of a peripheral artery. Although these tumors do present in advanced stages, primary resection for palliation followed by surveillance and more localized treatment of metastatic lesions provides good symptomatic relief and avoidance of arterial embolization. In this case cytotoxic chemotherapy was not given because of the good palliation from surgery and radiation and the lack of any strong data to support chemotherapy in slow-growing myxoid leiomyosarcomas.
We recommend that aggressive tumor extirpation be the operation of choice for intraluminal leiomyosarcomas and not radical aortic resection. The goal of surgery is not cure but palliation. Local recurrence was not seen, and the tumor was easily removed. Subsequent aggressive radiation and fixation of other metastatic lesions are also warranted by the potential of long survival. Extra-anatomic bypass could be performed, but the ease of removal and decreased risk of large embolization favor tumor endarterectomy over bypass.
References
This article has been cited by other articles:
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  B. P. Van Putte, T. L. Bollen, and M. A.A.M. Schepens Bleeding sarcoma of the aorta mimicking a symptomatic aneurysm J. Thorac. Cardiovasc. Surg., June 1, 2007; 133(6): 1643 - 1644. [Full Text] [PDF]
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